ABSTRACT.Purpose: To study the course and visual outcome of Vogt-Koyanagi-Harada (VKH) syndrome in children. Methods: The medical records of 97 consecutive patients with VKH syndrome were reviewed. Patients had been seen at King Khaled Eye Specialist Hospital in Riyadh, Saudi Arabia. The records were divided into two groups: those in whom the onset of the disease occurred at the age of 14 years or younger (pediatric group) and those in whom the disease manifested after the age of 14 years (adult group). Results: Eight (61%) of the 13 children required cataract surgery compared to 14 (17%) of 84 adult patients. Despite medical and surgical therapy, eight (61%) of the pediatric group had a final visual acuity of 20/200 or worse compared with 22 (26%) of the 84 adult patients (pΩ0.0103). Conclusion:The course of early-onset VKH (before the age of 14 years) appears to be aggressive. The ocular complications are more severe in children than in adults, leading to rapid deterioration in vision. Despite therapy, the final visual outcome of VKH in children is poor.

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The present study was undertaken because of the recent report of an association between familial multiple sclerosis (MS) and prior ownership of a small pet. Forty-five patients with sporadic MS were retrospectively compared with 45 closely matched controls and were found to have owned a significantly greater number of indoor dogs prior to the onset of MS. Importantly, the MS patients had also had more exposure to neurologically ill dogs in the five years before the onset of symptoms. These new data further support the hypothesis that house dogs may serve as an animal vector in MS.

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Rosai-Dorfman disease (RDD) is a rare histiocytic disorder most often characterized by painless cervical lymphadenopathy, but it may also present with orbital disease. The clinical course of RDD is variable; it can be either relapsing-remitting or progressive, and the outcome relates to clinical location and treatment response. Orbital RDD can have an insidious onset and similar presentation to other ophthalmic conditions; this can result in a delayed diagnosis. Nearly all cases of orbital RDD cause visual disturbances and require treatment. Because orbital RDD is an uncommon presentation, a variety of interventions have been employed, including surgery, immunotherapy, chemotherapy, and radiotherapy. We present a case of salvage radiotherapy for progressive orbital RDD refractory to surgery and chemotherapy in a pediatric patient.

Three children from consanguineous parents began losing the ability to walk in late infancy. Despite chronically progressive weakness leading to wheelchair dependence by adolescence and later loss of motor speech production, intellect remained preserved. Examination revealed upper motor neuron findings of pseudobulbar palsy and spastic quadriplegia, without dementia, cerebellar, extrapyramidal or sensory signs. In addition they exhibited a diffuse conjugate saccadic gaze paresis, especially severe on down-gaze. CT and MRI scans of brain and spinal cord, EEGs, visual and brainstem auditory evoked potentials, CSF examinations, enzyme assays for lysosomal storage diseases, blood amino acids and urine organic acids were all normal. Cortical somatosensory evoked potentials were poorly configured in two of the patients, though they had normal central conduction. EMG showed no signs of denervation. Nerve conduction studies showed normal peripheral motor and sensory conduction velocities. Transcranial magnetic stimulation of the brain elicited no motor-evoked potentials. Despite the lack of neuropathological confirmation, the clinical course and neurophysiologic data strongly support the diagnosis of a familial (autosomal recessive) primary lateral sclerosis (PLS).

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Any thyroid cancer can metastasize to the uveal tract, even after decades; medullary thyroid cancer can be part of multiple endocrine neoplasia syndrome. Superior limbic keratoconjunctivitis and lagophthalmos are prognostic markers for more severe thyroid-associated ophthalmopathy (TAO). The restrictive ophthalmopathy of TAO may be associated with more sustained ocular hypertension and require topical therapy. Several new studies address the therapy of TAO, ranging from retrobulbar to oral to intravenous glucocorticoids, alone or combined with radiotherapy. Endonasal decompression of the posterior orbit can be done well for severe optic nerve compression; however, leaving the anterior orbital septum intact can minimize postoperative diplopia. Smoking increases the risk and relapse rate for ophthalmopathy. Thyrotropin receptor antigen on fibroblasts diffusely in the body is causative in TAO and pretibial myxedema with even increased urinary secretion of glycosaminoglycans. Corticosteroid-responsive patients show a sustained up-regulation of the Th1/Th2 profile.